Monday, August 31, 2009

When I first opened my eyes this morning, all I wanted to do was cover my head and stay put all day long. We had an appt. with a man from the funeral home. So, get up, get a shower, don't think about it, drink coffee, don't think about it, check on Bri, don't think about it...walk the dog, don't think about it.....he pulls into the yard and oh, there's no words. Thank goodness he wasn't driving a vehicle marked accordingly. I asked him when I called last Friday if the vehicle he would be coming over in would have any type of business identification on it and he said no. Thank God for little things.

Mr. Dan was such a nice, respectful christian gentleman. He stated that he knew how we felt because he and his wife also had a child pass away at an early age. I was ok until I had to fill out a form with Brianna's information. That's when the tears started to flow....Right now we are planning on a Memorial Service and not a funeral service. There is absolutely no way that neither Jerry or I could stand to see a child-size casket. I don't think I could even walk inside the church without losing my mind. Anyway, we made it though the preliminaries.

The stress we are under makes simple ordinary things HUGE. I can't seem to stop crying, I don't want to be in the house waiting for the time but nor do I want to be away from the house and lose precious time. I say mean things to Jerry, I find myself isolating myself, I can't quite explain it except to say that it hurts so bad. I want this chapter in my life to be over yet I don't want it to be over. And for all of you T-18 moms out there whose children are alive, if you are like I was before this pulmonary hypertension death sentence, I hung on every word other T-18 mothers said hoping and praying that what they were going through would not be what I would be going through one day. I always wondered exactly what would take Brianna's life, a cold turned to pneumonia, cancer, just what would it be. All I have to say now is that it could be worse. As far as we know Brianna is not in any pain. That is our continued prayer at this time. No pain for sweet baby Bri.

Sunday, August 30, 2009

A day to give thanks because we are enjoying yet another day with our little miracle baby girl, our older daughter Ashley and Grandmother. Bri's first venture out in a week to church this morning and we enjoyed every minute of it. Oh, to look at Brianna is so deceiving. She presents so healthy and happy yet it is her heart and lungs that are giving out. Thank you a hundred times over to our spiritual family at FPCO. Your support has been life-saving, really.

Saturday, August 29, 2009

That's how I feel. Running on auto-pilot. Did I tell you that Brianna can't have baths anymore? Nor can she go swimming in any body of water. Well, actually she can if we want to end her life sooner than later. Sounds crazy but the one common denominator in all three major "blue" spells was that they happened within 10 minutes after a bath. When Jerry said after the second one, no more baths, I was like what? That's ridiculous but actually it's not. When Brianna is placed in warm water, it causes her body to adjust to the temperature which changes her blood flow, which causes the heart to send un-oxygenated blood throughout her body, so she is still breathing, but there is no oxygen. Which explains why during the last blue spell, she was breathing from the oxygen tank yet she stayed blue for at least 10 minutes. So now it is wash-cloth baths all the way.

We have enjoyed the many visitors. Here's some pics I snapped. Didn't get one of everyone. Oh well....

Friday, August 28, 2009

It's heartbreaking to see how tired Brianna is now. She has been taking cat naps periodically through the day yet when she is awake, she is her usual happy, rolling, sitting up self. Her little body is getting so tired.

We almost got through a night without alarms until 3:04 am....and then today I was on the phone with a long distance dear friend, Martha Bravo (the photograph who has taken the most amazing pics of our family) when Brianna's heart rate spiked to 256. In total shock I hung up on Martha and concentrated on Brianna (with Jerry). It came right down but boy did I feel like I needed a stiff drink after that one!!!

We had a very good visit with our friends Steve, Ibis, Jim, Karen, Vance and Theresa this evening and they brought over a wonderful "comfort food" dinner. Good friends, good food and good times. Thanks to you all!

It's been a very difficult week. Today Jerry had Bri's pediatrician sign the DNR (Do Not Resusitate) form. Learned something new about this. If by chance we were out and Brianna had an episode and an innocent bystander called 911, without us having the original DNR form on us, the EMS would HAVE to do whatever is necessary to keep Brianna alive. We would have no say whatsoever if we did not have the original with us.

I also called the funeral home to arrange for a meeting next week. This stuff is so unbelieveably hard, I guess by blogging about it, it helps me....sounds crazy I know. It is crazy and so very wrong.

Thursday, August 27, 2009

We all had a good night last night. Brianna's pulse oximeter didn't alarm not one time...yippee!!! We actually heard her playing with the sweet seahorse light up toy that her friend Maddie brought over to her earlier in the evening. It's the kind of thing that wakes you up from sleeping but ever so lightly and makes you smile with delight because your sweet baby is enjoying life. Thank you Kristen and Maddie for making that possible : ) Hubby is in bed, Grandmother is in Bri's room watching over her and I am waiting for all to go to sleep so I can go to sleep. Thank you to everyone for the many thoughts, prayers and food!!!! I have never been in this position and it feels kinda ackward but at the same time, feels so good not to have to think about meal planning, shopping and cooking. I have new pics but too tired to upload them now. Tomorrow I will attempt that. God has been so good to us. As for a prayer request, pray for no pain for sweet Brianna.

Wednesday, August 26, 2009

There are many times in ones life that things are put before them and they say NEVER! and someone usually pipes up, never say never. Well, not too long after Brianna was born, Ashley put in to have a tattoo (her first and hopefully her last)) of something to pay tribute to Brianna. We've talked about it many times but kept putting it off. Well, now that Brianna is not doing very well, Ashley asked again....and after deliberation we said yes, ONLY after reviewing the artwork prior to the tattoo. (We have also kept in the back of our minds that she is 17 1/2 and if she got a tattoo now, we would have some say in it, if we waited 6 more months....yikes. Who knows what she might do. So....today was the day and I have to say that I really like it. Later this evening she shared with me that now Brianna will be with her always, somehow the tattoo makes Ashley feel that Brianna is even closer to her heart. Sweet.

Curious?......

Brianna also had a special visitor this evening, her friend Maddie. Isn't she the cutest?!!! She kept reaching over to hug and kiss Brianna.

Brianna is doing good. The oxygen has made a big difference; giving her comfort. She episodes continue from one extreme to another. Sometimes they are the super-high heart rate; blue spells and then other times, the extreme low heart rate. This morning at 3 am it was the extremely low heart rate. We have learned that it at those times she is experiencing a Bundle Branch Block (google it, easier to understand). Several hours later she is back to "normal".

Thank you again to everyone who is sending emails; texts; notes on Facebook and comments on this site. Although I don't respond to each one of you, your thoughts, prayers and kind words do not go unnoticed. Thank you from both Jerry and I.

My momma is here in town now. My sister Judy brought her. A super big thank you to her. Everyone knows in times of sadness and distress, it's always good to have your mom around.

Monday, August 24, 2009

Once again Vera's mum, May, had something on Vera's site that I just HAD to copy and paste. Hope you don't mind May. God's blessings to your family.

THESE MOMS SHARE SPECIAL GIFTS

by Erma Bombeck

Most women become mothers by accident, some by choice, a few by social pressures, and a couple by habit. This year, nearly 100,000 women will become mothers of handicapped children. Did you ever wonder how mothers of handicapped children are chosen?

Somehow I visualize God hovering over Earth selecting his instruments for propagation with great care and deliberation. As he observes, he instructs his angels to make notes in a giant ledger.

Finally he passes a name to an angel and smiles, "Give her a handicapped child."

The angel is curious, "Why this one, God? She's so happy."

"Exactly," says God. "Could I give a handicapped child a mother who does not know laughter? That would be cruel."

"But has she patience?" asks the angel.

"I don't want her to have too much patience, or she will drown in a sea of self pity and despair. Once the shock and resentment wear off, she'll handle it. She has that feeling of self and independence that is so rare and so necessary in a mother. You see, the child I'm going to give her has his own world. She has to make him live in her world, and that's not going to be easy."

"But, Lord, I don't think that she even believes in you."

God smiles, "No matter. I can fix that. This one is perfect. She has just enough selfishness."

The angel gasps, "Selfishness? Is that a virtue?"

God nods, "If she can't separate herself from the child occasionally, she'll never survive. Yes, here is a woman whom I will bless with a child less than perfect. She doesn't realize it yet, but she is to be envied. She will never take for granted a spoken word. She will never consider a step ordinary. When her child says Momma for the first time, she will be present at a miracle and know it! When she describes a tree or a sunset to her blind child, she will see it as few people ever see my creations."

"I will permit her to see clearly the things I see...ignorance, cruelty, prejudice...and allow her to rise above them. She will never be alone. I will be at her side every minute of every day of her life, because she is doing my work as surely as she is here by my side."

"And what about her patron saint?" asks the angel, pen poised in midair.

At 3:15 am I woke with a start, looked at the baby monitor and something didn't feel right so I went to Bri's room. She was restless, rolling back and forth, moaning a little bit. When I picked her up, she was warm, clammy, sweaty. Her oxygen level was good but I noticed that her heart rate was climbing and climbing and climbing and although she was just laying in her bed, she was breathing like she had just finished running a race. The monitor alarms when her heart rate reaches 160, well, it alarmed, I pressed the silent button (works for 60 seconds), alarmed again, silenced it again, alarm, well, that is how it went for quite some time. By the second alarm Jerry had joined me in her room. We were giving her oxygen but that did nothing to slow down the heart rate. There we are sitting in the dark, on the floor, holding Brianna, praying, praying, praying that if it is her time, take her quickly and without pain. After an hour of the three of us on the floor, two of us obsessed with the numbers on the machine, the third being just trying to slow down from the "race", we negotiated. I laid down for an hour while Jerry kept vigil, then Jerry slept while I kept vigil. After several hours of the high heart rate (into the 170's) Brianna rested.

We have kept the oxygen on her continuously since then. There were several times today when it appeared that she was starting to have an episode but then it subsided.

Thank you to the many friends who came by to visit with us today. We feel blessed that God has given us this time to prepare (yeah, right, huh) and for our many friends and family to visit with Brianna while she is still here on this earth. She continues to be a happy, smiley little girl. God has been so good.

On another note, wonderful news. We have often talked about the idea of starting a non-profit organization to help families like ours, receive respite care. Sadly, there are too many families who don't qualify for any government/organization programs to help families cope with situations like ours. We plan to change that. Our organization is: Brianna Gives Hope. We will be posting more information as it developes. Many thanks to those who helped us make it a reality. We are looking forward to our first Golf Tournament and Motorcycle Poker Run.

Anyway, Brianna is in bed, I am watching her on the monitor and listening to her babble (while I also listen to my husband snore...lol. God bless that man. My heart breaks for him. His only natural daughter, and he has to watch her die. Breaks my heart.

The oxygen has given Brianna new strength. For how long we don't know but we will take whatever we can get.

Here's Brianna on her swing last week. Oh how she loves the wind in her face.

Saturday, August 22, 2009

We are home from the hospital. Met with the cardiologist today. Brianna's pulmonary hypertension is so severe that there is nothing more that can be done in her particular case. We have chosen comfort care at this time. Brianna will be on oxygen to help alleviate the tightness in her chest. Of course we don't know what God's plan is at this time but we do know that God has a plan for every single one of us and the amount of time we will be here on this earth. Faith. We must continue to have faith. As I have said before, we continue to be grateful to God for allowing us to be the caretakers of Brianna for this time. I think I can rightly boast that God must be proud of us for how well we have taken care of her because he has continued to allow her to bless our lives.

It is with tremendous sadness that I let everyone know that Brianna is in the hospital again. Yesterday she turned blue again. Just started having problems getting enough oxygen, I immediately hooked her up to her pulse oximeter and her blood oxygen levels were at 61 (should be 100), got the oxygen going and even with that, her levels wouldn't increase. She was moaning and trying to breath, turning pale and blue. Jerry was home with me at the time, we continued with the oxygen hoping the episode would pass but after 8 to 10 minutes we called 911. When the paramedics arrived, they took one look at Bri, saw the blood oxygen level, and the oxygen and her little blue face and bolted us out the door into the ambulance on to the hospital. They were preparing to intubate her in the ambulance when her oxygen dropped again but thank you God, her levels came up.

We were in the ER for a while and then she was admitted. Today we have found out that her pulmonary hypertension is extremely severe. I have noticed in the last month that her heart has become larger due to having to work extra hard to push the oxygen into her lungs. The first indication of a problem was the bundle branch block that happened last week. We know there are meds for hypertension but the problem is she needs a cardiac catherization before the proper dosage of meds can be administered or they could be fatal. Last week her cardiologist told us he could not do the cathe. We are now meeting with a new cardiologist but the fear is that the procedure would be very high risk and she would not survive it, that is even if we could find a cardiologist who would agree to do it. Even if so and she survived it, how much time would the meds give her to live? Then we have to ask ourselves if we are being selfish in putting her through this? We want her here, we want her to stay with us. You can't imagine how hard this is.

We finally allowed ourselves to really look to the future, a teacher from the school would start coming to the house in the Fall, and when she turned 3 she would be going to school, we ordered her a wheelchair, I've been planning her Halloween costume. There are so many photos I still want to take, places we want to take her.

Please pray, pray, pray for just a little more time. Just 5 minutes more.....

Thursday, August 20, 2009

Oh, I am so looking forward to tomorrow, or should I say later today since it is 1:27 am. Why am I still awake? Because Bri is and she threw up not to long ago so I want to make sure she is asleep before I crash. Anyway, back to later today....I have TWO, yes TWO volunteers coming to watch Brianna. One is a volunteer from our church, Cassie is her name. She has pediatric nursing experience and is going back to school for her masters degree. The other volunteer is from Hospice, she is also a nurse or actually a retired nurse. Either way, I'll take both of them. It was coincidence that they both were available only on Thursday this week but heck, I didn't want to miss the opportunity. What will I do with my time? Hmmmm.......will let you know later.

Saturday, August 15, 2009

Looks are deceiving and what I mean by that is when one looks at Brianna, they probably see a "special" child but they also see a "healthy" child. "Healthy" is far from the truth. Every second of every day that Brianna continues to breathe is a gift from God. And now, more than ever. How do we do it? We live each and every day to the fullest. Really. Today I had soooo many chores to do but guess what, I sat on the floor with Brianna and sang songs, danced and played. Held her as close to me as I could, breathing in her scent. The chores could wait. She couldn't. I took her outside and pushed her in her swing. Took lots of smiley photos (but can't locate the cable to connect camera to computer right now....tomorrow, hopefully) Brianna is just like me and her dad, she LOVES fun, crazy things. Her favorite on the swing is to go round and round.....it's hard to be sad when you look at her smiling, happy face. We are so blessed.

Thursday, August 13, 2009

After Brianna was born our life as we knew it completely changed. For a while we were on a horrendous roller coaster ride that seemed to level off for a while. We felt like we were flying "under the radar". No more. This last month and a half has been quite stressful. Brianna has turned blue three times which means three hospital stays, 3 EKGs, 2 EEGs, 3 chest x-rays, not to mention other tests. The latest episode was this evening. Now we are up to 4 hospital trips in 6 weeks. The past couple of days Brianna has been lethargic and very sleepy and she has been very pale. Today, after an outing with Ashley, we came home and put Brianna down for her nap, at which time we had noticed that her coloring had been off for a couple of hours. So, after coming home and laying her down, we hooked her up to the pulseoximeter to measure her O2 and heart rate. O2 was good but for the first time, her heart rate was 68-75. Not a good feeling for us. Normal rates are 105-110 with a sleeping rate in the 90's. I continued to monitor the rate for over an hour and it stayed low. Too low. We started to become quite nervous. I woke her up and tried to get her heart rate up but as soon as it went up to 100 or so, it immediately dropped back down to the 70s. We were very, very worried. This low heart rate thing is new to Brianna and to us.

Background: After turning blue last week when a Ph Probe test was being administered, the hospital ran some tests. Yesterday I received a call from the Pulmonologist that was very upsetting. The tests administered last week show extreme pulmonary hypertension in Brianna's lungs. We were advised to meet with the cardiologist immediately. The Nissan Fundoplication procedure we had hoped to have done (to stop the reflux coming up and flowing into her lungs) cannot be done now due to the severity of the pulmonary hypertension. At least not until a cardiac catherazation is performed. I then called Brianna's cardiologist and heard heart-breaking news. The cardiologist told me directly that he was sorry but there was nothing else he could do for Brianna. I felt like the rug had been pulled completely out from under me. Where do we go from here? Just sit and watch our daughter die a slow death from her body's inability to draw enough oxygen due to the high pressure in her lungs? Here it was, the moment we have heard about from other T-18 families, doctors reaching a point to where they feel there is nothing else that they can do. Is it because he does not think she would survive the cardiac catherazaion and does not want the burden of her death....I don't know. All I know is we now are at a place where we truly have to decide when enough is enough. How much do we put Brianna through? Are we being selfish? What is her quality of life at this point? What will it be in the next month? Do we find another cardiologist, etc. I could go on and on.

Decision: Meet with a new cardiologist at a new hospital, meet with Brianna's entire team of docs and go from there. I wrote the above posting late last night but wanted to re-read it today when I wasn't so tired and emotional. Today, Brianna has been her usual smiling happy self. We found out that the low heart rate was ok. It is associated with heart disease. It might happen again and it might not.

Saturday, August 8, 2009

We returned home Friday evening around 6 pm. Yesterday ended up being a very productive day. Usually we go to Florida Hospital (which we like) but the doctor who ordered the Ph Probe is associated with Arnold Palmer Hospital for Children so that is where we were for the last couple of days. It was good to see different doctors and to hear what they think is going on and what we should do. One doctor in particular, Dr. S was absolutely amazed to see Brianna. She is the oldest Trisomy 18 child he has ever seen. And what a show she put on for him. Goofy, silly, rolling around, sitting up, babbling, the whole 9 yards! He was mesmerized. We ended up doing another EKG and EEG, chest x-ray and a stomach emptying procedure (formula and a dye and put into her stomach through her G-tube and she laid under a scanner that recorded how long it took for her stomach to empty. She laid there an hour and a half, smiling, clapping and laughing at the many cartoon stickers on the scanner. She is so darn adorable.

We will get the results on Monday. We are still on the path of doing the Nissan Fundoplication but we also want to rule out other problems while she is in the hospital. As my husband says, it would be frustrating to do the Nissan only to find out it was something else causing the blue spells.

Here's a picture of Bri getting all wired up for an EEG. She was so patient.

After getting the wires connected, her head is wrapped to keep them in place. I HAD to add the bow.

Thursday, August 6, 2009

Good news: spot on Bri's neck does not appear to be MRSA. Must be a bug bite. Whew. Semi Bad news: we went to the hospital for the Ph Probe, which is supposed to be a no-big-deal procedure but not for little Bri. In her nose and down her throat went the thin tube and about 8 minutes later, Bri started turning blue. I think it frightened the Endoscopy nurse more than it did me. So, out came the tube and in came the doctor, nurses, respiratory, etc. After getting her back to pink and rosy, it was decided to cancel the Ph Probe but they still want Bri to stay overnight for observation.

Where do we go from here? Not 100% sure but we are talking with Bri's pediatrician and the doctors here to come up with a plan. It is very possible that we will proceed with the fundoplication procedure and hopefully that is what is needed to stop the blue spells although no one has been convinced 100% that reflux is the culprit.

Wednesday, August 5, 2009

I know that I have been needing to post but to be honest I have been so very tired. Let's see, where do I begin, so, we drove home from Pensacola after getting an oxygen tank for the "just in case Bri turns blue while driving home" and I must say during that drive I decided that I HAD to contact Hospice again. Not because I feel her life is ending soon (oh, how I hope not) but because I need the services they offer before I lose my mind, really. What a God-send calling Hospice has been. The team that we were assigned to is the Yellow team (my favorite color) and oh, what a wonderful team it is. Bri's nurse, Kara, I felt such a connection from the moment she walked into the room. Then there was the Medical Director, who remembered Bri from the first time we had signed up for Hospice, he was so happy to see her, just the look on his face, the way he talked, he is so proud of our little angel pie. And today I had the opportunity to meet Elizabeth, the social worker, with Hospice. We clicked like nobody's business. I feel like God has personally picked this team for us. Thank you God.

But moving on, we haven't had any more "blue" spells. We are scheduled for a Ph Probe tomorrow at Arnold Palmer Hospital, it requires an overnight stay. There will be no sedation involved. Only monitoring by nurses. Big relief. There is just one very BIG thing that might keep this from happening. Like life has not been crazy enough, on Monday night before putting Bri to bed I noticed a pink nickel-sized spot on her neck. Me being the dork that I am, I retrieved the flashlight and magnifying glass to check it out. Didn't see anything that resembled a sting or bite so.....fast forward to Tuesday morning. OMG....the spot on Bri's neck is now the size of an orange, inflamed, and then inside of that, the size of a quarter, red/purple. Off to the pediatrician we went. One look and he is concerned that it might be MRSA. Big, big, big scare. He immediately put Bri on antibiotics specific for that and topical ointments. We have to return in the morning for a follow-up. I am so hoping it is a bite of some sort and not MRSA. If it is MRSA, no Ph probe tomorrow. Not sure if the antibiotics will work on MRSA or if she will have to be hospitalized for stronger meds. Will post tomorrow and let you know.

On another note, Ashley visited with us for a day and a half. What a nice visit we had. I miss that girl.

Our Family Christmas 2008

Photo by Martha Bravo

Definition of Trisomy 18

Edwards Syndrome : Edwards Syndrome (Trisomy 18) is a chromosomal abnormality where there is an extra chromosome present in every cell of the body. This is an example of where “the more the merrier” is not true. This extra chromosome means that every cell has extra information encoded into it. The extra information causes confusion in the way that the cells are formed and results in the potential malformation of all of the body systems. Sadly, this condition is considered "not compatible with life". Just like with Down's Syndrome (Trisomy 21), there is a wide range of how this condition will play out (what the doctor's will refer to as your child's phenotype). Unfortunately because there is more information encoded on the 18th chromosome, the severity of this condition is greater than that of Down's Syndrome. Current studies show that while 1:1500 children will be diagnosed prenatally with trisomy 18, only half that number (or 1:3000) will be born alive at full term. Of those who survive to birth, only half will make it to two months of age, and only 10% will survive to their first birthday. Children who live can be an important part of their family and community, but are profoundly developmentally delayed.

But oh, how we love these special little babies. When Brianna was born we were told she would probably leave this earth within 24 hours...but look at her now. 24 months old, healthy, adorable and amazing. God has been so good. Brenda

Bri's Medical Condition

Brianna was full-term born on April 12, 2007, weighing 5 lb 18 oz and 18" long. Her Apgar scores at birth were 8 and 9. Approximately 15 hours after her birth we were advised of the tentative Trisomy 18 diagnosis.

Immediate health issues had to do with her heart: Mainly, a VSD (Ventricular Septal Defect); PDA (Patent Ductus Arteriosus) ; and a PFO.

Open Heart Surgery at 4 months and

G-tube placement before we came home.

Eye surgery in Spring of '08 to correct cross-eyes.

Tubes placed in ears in Fall of '08 hoping to quash the numerous ear infections.

Scan of abdominal section in Dec. '08. Everthing appears normal.

Brianna takes formula by g-tube but eats baby food by mouth. She can hear, see, is rolling over, sitting up, trying to crawl, playing with toys, says "da da"; "do it, do it, do it" and various other sounds. She is the happiest child we have ever known.